2001 CTOS Annual Meeting Posters— Surgery

TWO HUNDRED AND SEVEN EXTRA-ABDOMINAL AGGRESSIVE FIBROMATOSIS TREATED AT A SINGLE INSTITUTION: AN ANALYSIS OF OUTCOME.
Alessandro Gronchi¹,  Dario Baratti¹,  Elisabetta Pennacchioli¹,  Luigi Mariani²,  Salvatore Lo Vullo²,  Laura Lozza³,  Maurizio Colecchia⁴,  Paolo Casali⁵,  Rossella Bertulli⁵,  Mario Santinami^1
1Dept. of Muscolo-skeletal surgery. Istituto Nazionale per lo studio e la cura dei Tumori.,  ²Dept. of Biostatistics. Istituto Nazionale per lo studio e la cura dei Tumori.,  ³Dept. of Radiotherapy. Istituto Nazionale per lo studio e la cura dei Tumori.,  ⁴Dept. of Pathology. Istituto Nazionale per lo studio e la cura dei Tumori.,  ⁵Dept. of Medical Oncology. Istituto Nazionale per lo studio e la cura dei Tumori.

METHODS: From May 1966 to February 2001 207 pts (median age 36, M/F 51/156), affected by AF (arising from extremities in 41, girdles in 66, abdominal or chest wall in 56 and from other sites in 44), have been treated at our institution. 131 (median size 5 cm.) presented with primary disease, while 76 (median size 6 cm.) had had at least one local recurrence before to be referred. Surgery alone in 167 patients, while surgery + radiation therapy in 40 (20 for each group) have been the procedure performed at presentation. Quality of margins was reviewed by a single pathologist and 149 were found to be negative (99 for primary cases and 50 for recurrences), while 58 were microscopically positive (32 and 26 respectively). Both univariate and multivariate (Cox model) analyses of possible prognostic factors were performed.

RESULTS: During follow-up (median time 85 months) 55 patients recurred. Four patients died of local progression. The overall survival was 98% at 5 years and 95% at 10 years, while disease free survival was 71% at 5 years and 68% at 10 years. Primary cases had better outcome with 80% disease free survival at 5 years and 74% at 10 years. Recurrences had 56% disease free survival at 5 years, stable up to 10 years. In primary cases size (> 5cm.) and site (extremities and girdles) were found to be significant prognostic factors, while quality of margins did not affect outcome. In recurrences none of the prognostic factors analyzed were found to be significant in predicting local recurrence. Multivariate analysis confirmed the prognostic role of status at presentation (primary versus recurrence) and size. A borderline P was obtained for site and there was a suggestion that quality of margins could be prognostic only in recurrences.

CONCLUSION: At variance with sarcomas, quality of margins doesen’t predict outcome in primary tumors. Wide resection is still the cornerstone of treatment, but attempts to achieve negative resection margins may result in unnecessary morbidity and may not prevent local recurrence. Operations that preserve function and structure should be the primary goal, because the presence of residual disease cannot be clearly shown to impact adversely on 5 years and 10 years disease free or overall survival. Recurrences are at a higher risk of further recurrence, especially if operated with positive resection margins. Adjuvant radiation may be delivered in selected cases.